Cardiomyopathies

Question 1

What are the general classifications of cardiomyopathies?

Answer 1

Primary – due to an idiopathic process effecting the myocardium. Secondary – related to a specific heart muscle disease

Question 2

What are the functional classifications of cardiomyopathies?

Answer 2

Dilatated: ventricular enlargement and systolic dysfunction. Hypertrophic: inappropriate myocardial hypertrophyin the absence of HTN or aortic stenosis. Restrictive: abnormal filling and diastolic function

Question 3

What is dilated cardiomyopathy?

Answer 3

Ventricles stretch and become flabby and the myocardium deteriorates. Systolic dysfunction and pump failure with low cardiac output.

Question 4

What is the pathophysiology of dilated cardiomyopathy?

Answer 4

The heart’s attempt to work harder results in increasing levels of Ca2+ in the cardiac cells. This activates a calcium-sensitive enzyme initiating a cascade which switches on genes that cause heart enlargement

Question 5

How do you differentiate dilated cardiomyopathy and dilated left ventricle due to severe CAD?

Answer 5

Usually post MI - local dysfunction, Dilated myopathy - global dysfunction

Question 6

What is the clincial presentation of IDCM?

Answer 6

Heart failure symptoms, Anginal chest pain, Emboli, Syncope, Sudden cardiac death and Arhythmias

Question 7

How is dilated cardiomyopathy diagnosed?

Answer 7

CXR- enlarged heart, biventricular enlargement, and pulmonary vascular congestion. EKG- LVH, Left atrial enlargement, Q waves, poor R wave progression, Afib. Echo: ventricular enlargement, increased systolic and diastolic volumes, decreased EF

Question 8

What is treatment for DCM?

Answer 8

Limit activity, Salt restriction, Fluid restriction. Medical: ACE inhibitors, diuretics, digoxin, hydralazine / nitrate combination, Anticoagulants, Anti-arrhythmics

Question 9

What is peripartum cardiomyopathy?

Answer 9

Ventricular dysfuntion developing in the last month before delivery to 5 months postpartum

Question 10

What are risk factors for peripartum cardiomyopathy?

Answer 10

Advanced maternal age, African-American race, Multifetal pregnancies, Preeclampsia, Gestational HTN

Question 11

What is hypertrophic cardiomyopathy?

Answer 11

Inappropriate, asymmetric myocardial hypertrophy. Septum is usual site for hypertrophy. Cells with bizarre appearance. autosomal dominant and most common cause of young sudden cardiac death

Question 12

What is the suggested pathology of hypertrophic cardiomyopathy?

Answer 12

Normal myocytes react to excessive mechanical loads with the release of autocrine growth factors. In HCM, normal systolic pressures sensed by abnormal myocyte as excessive load, initiating further hypertrophy. Sarcomeric dysfunction

Question 13

What are symptoms of hypertrophic cardiomyopathy?

Answer 13

HTN, dyspnea on exertion, angina like chest pain, palpitations, syncope, sudden death

Question 14

What will you find on physical exam with hypertrophic cardiomyopathy?

Answer 14

Apex localized, Palpable S4, Harsh systolic ejection murmur across entire precordium → apex & heart base

Question 15

What are the cardiac findings associated with hypertrophic cardiomyopathy?

Answer 15

LVH. deep and narrow Q waves. Asymmetric septal thickening. Systolic anterior motion of anterior mitral leaflet. Ejection murmur LSB. Often mitral regurg murmur. S4

Question 16

What is the management for hypertrophic cardiomyopathy?

Answer 16

All first degree relatives: screening echocardiography/genetic counseling. Avoid competitive athletic/discourage rigorous exercise. Holter x 48 hours (sudden death comes from arrhythmias)

Question 17

What is the medical treatment for hypertrophic cardiomyopathy?

Answer 17

Vasodilators, Inotropes, Diuretics, B-blockers for longer diastolic filling time

Question 18

What is treatment for non responsive hypertrophic cardiomyopathy?

Answer 18

Surgery (Myotomy/Myectomy), ICD, DDD pacemaker, Non-surgical septal reduction

Question 19

What is restrictive cardiomyopathy?

Answer 19

Diastolic dysfunction: the ventricular walls are excessively rigid & impede ventricular filling. Myocardial fibrosis, hypertrophy, or infiltration due to variety causes. The atria frequently become enlarged

Question 20

What can cause restrictive cardiomyopathy?

Answer 20

Deposition of abnormal substances in the myocardium. Infiltrative disease: the abnormal substance is largely between the myocytes–>Amyloidosis, Sarcoidosis. Endomyocardial: fibrosis or radiation

Question 21

What are clinical features of restrictive cardiomyopathy?

Answer 21

Exercise intolerance & dyspnea, dependent edema, ascites, anasarca, enlarged, tender, & often pulsatile liver. Jugular venous distension, S3, S4

Question 22

What is used for diagnosis or restrictive cardiomyopathy?

Answer 22

CXR-signs of CHF without cardiomegaly. EKG-nonspecific changes most likely. ECHO-thickened LV walls with normal or slightly decreased volume

Question 23

What is amyloid heart disease?

Answer 23

Thickened, firm, rubbery ventricular muscle. Major diastolic dysfunction. Abnormal Q waves – look like an M.I. Conduction disturbances, arrhythmias. thickened walls everywhere

Question 24

What is the treatment for amyloid heart disease?

Answer 24

Drug therapy must be used with caution diuretics for extremely high filling pressures. Vasodilators may decrease filling pressure. Calcium channel blockers to improve diastolic compliance

Question 25

What is myocarditis?

Answer 25

Involvement of the heart with an inflammatory process. Direct damage, toxins, or immunologic response. North America: viral. South America: Trypanosoma cruzi

Question 26

What are the findings in myocarditis?

Answer 26

Cardiac symptoms and signs following flu like syndrome. Fatigue, dyspnea, palpitations. Pericardial angina-like pain. Tachycardia. S3 and mitral regurg murmur. Severe = congestive heart failure

Question 27

What is treatment for myocarditis?

Answer 27

Bed rest. If CHF evident: diuretics, oxygen, inotropes like digoxin